Cardiac light-chain deposition disease relapsing in the transplanted heart

Aimo, Alberto; Vergaro, Giuseppe; Pucci, Angela; Bernazzali, Sonia; Maccherini, Massimo; Buda, Gabriele; Passino, Claudio; Merlini, Giampaolo; Emdin, Michele

doi:10.1080/13506129.2017.1334196

Cited by 5 publications

(2 citation statements)

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“…Lambda light chains represent the most common amyloidogenic precursors in AL amyloidosis. In such cases, positive immunostaining only for the lambda light chain is detected, i.e., the so-called monotypic restriction [ 10 , 15 , 18 , 28 , 29 , 54 , 55 ] ( Figure 6 and Figure 7 A).…”

Section: Immunohistochemistry Of Cardiac Amyloidosismentioning

confidence: 99%

Tissue Characterization in Cardiac Amyloidosis

et al. 2022

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Cardiac amyloidosis (CA) has long been considered a rare disease, but recent advancements in diagnostic tools have led to a reconsideration of the epidemiology of CA. Amyloid light-chain (AL) and transthyretin (ATTR) amyloidoses are the most common forms of cardiac amyloidosis. Due to the distinct treatments and the different prognoses, amyloid typing is crucial. Although a non-biopsy diagnosis can be obtained in ATTR amyloidosis when certain diagnostic criteria are fulfilled, tissue characterization still represents the gold standard for the diagnosis and typing of CA, particularly in AL amyloidosis. The present review focuses on the status of tissue characterization in cardiac amyloidosis, from histochemistry to immunohistochemistry and mass spectrometry, as well as on its future directions.

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Section: Immunohistochemistry Of Cardiac Amyloidosismentioning

confidence: 99%

Tissue Characterization in Cardiac Amyloidosis

et al. 2022

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“…In contrast, kidney transplantation is not considered an option in end-stage renal disease, as further kidney damage cannot be prevented in the transplanted organ [ 36 ]. In case of cardiac LCDD, LC deposits were identified as early as 3 months after heart transplantation [ 37 ].…”

Section: Discussionmentioning

confidence: 99%

Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease

Lestelle,

Beigelman,

Rotzinger

et al. 2024

Respir Res

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Background Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. Study design and methods A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. Results Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was < 0.70 in 45% of patients. Mean (± SD) FEV1 and DLCO were 86% ± 26.2 and 52% ± 23.9, respectively. CT revealed peculiar patterns of thin-walled cysts (58%) and thin-walled cystic bronchiectases (27%). Increased serum kappa light chain was found in 87% of patients. Histological analysis showed kappa light chain deposits in all patients, except one with lambda chain deposits. Median annual FEV1 decline was 127 ml (IQR 178) and median DLCO decline was 4.3% (IQR 4.3). Sixteen patients received immunomodulatory treatment or chemotherapy; serum light chain levels decreased in 9 cases (75%), without significant improvement in FEV1 (p = 0.173). Overall, 48% of patients underwent bilateral lung transplantation. Transplant-free survival at 5 and 10 years were 70% and 30%, respectively. An annual FEV1 decline greater than 127 ml/year was associated with increased risk of death or transplantation (p = 0.005). Conclusions Diffuse pulmonary LCDD is characterised by female predominance, a peculiar imaging pattern with bronchiectasis and/or cysts, progressive airway obstruction and severe DLCO impairment, and poor outcome. Lung transplantation is a treatment of choice.

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